Brief Article
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World J Gastroenterol. Jul 21, 2013; 19(27): 4356-4362
Published online Jul 21, 2013. doi: 10.3748/wjg.v19.i27.4356
Gastrointestinal side effects in children with Wilson's disease treated with zinc sulphate
Anna Wiernicka, Wojciech Jańczyk, Maciej Dądalski, Yesim Avsar, Hartmut Schmidt, Piotr Socha
Anna Wiernicka, Wojciech Jańczyk, Maciej Dądalski, Piotr Socha, Department of Gastroenterology, Hepatology and Malnutrition, the Children’s Memorial Health Institute, 04-730 Warsaw, Poland
Yesim Avsar, Hartmut Schmidt, Klinik und Poliklinik für Transplantationsmedizin, University Hospital of Muenster, 48149 Muenster, Germany
Author contributions: Socha P designed the study; Wiernicka A was responsible for the review of the literature, interpretation of data and initial preparation of the paper; Dądalski M analyzed the data; Schmidt H performed the genetic analysis; Jańczyk W and Avsar Y were involved in editing the manuscript; Avsar Y and Socha P prepared final version of the manuscript.
Correspondence to: Piotr Socha, Professor, Department of Gastroenterology, Hepatology and Malnutrition, the Children’s Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland. p.socha@czd.pl
Telephone: +48-22-8157384 Fax: +48-22-8157382
Received: October 15, 2012
Revised: February 6, 2013
Accepted: March 6, 2013
Published online: July 21, 2013
Processing time: 288 Days and 15.4 Hours
Abstract

AIM: To investigate the side effects of a zinc sulphate therapy in a cohort of Polish pediatric patients with Wilson’s disease.

METHODS: We retrospectively analyzed a cohort of 53 pediatric patients with Wilson’s disease treated at the Children’s Memorial Health Institute in Warsaw, Poland between the years 1996 and 2011 with zinc sulphate. Patients were diagnosed with Wilson’s disease according to the scoring system of Ferenci, with 49 cases confirmed by mutation analysis. Data about the dosage scheme of zinc sulphate, side effects and efficacy and toxicity of the treatment were collected and recorded in the patient’s medical chart at each visit to the hospital.

RESULTS: Mean age of diagnosis for the entire cohort was 10 years (range, 2.5-17 years). Duration of treatment with zinc sulfate was 83.3 wk (range, 8-344 wk). Side effects, all of gastrointestinal origin, were observed in 21 patients (40% - 9 males and 12 females), irrespective of the duration of therapy. Thirteen out of 21 patients were over the age of 10 years. The most common ATP7B mutation was p.H1069Q. Esophagogastroduodenoscopy, performed in 7 patients (33.3%) suffering from persistent and severe abdominal pain, revealed gastrointestinal ulcerations or erosions with negative Helicobacter pylori tests in all subjects investigated. The above mentioned 7 patients were treated with proton pump inhibitors. Three of those experienced resolution of symptoms, whereas proton-pump inhibitors failed to alleviate symptoms of the remaining four children and conversion of therapy to D-penicillamine was needed.

CONCLUSION: Zinc sulphate appears to cause significant gastrointestinal side effects, which children on therapy for Wilson’s disease should be closely monitored for.

Keywords: Wilson’s disease; Zinc; Abdominal pain; Gastrointestinal ulcer; Therapy

Core tip: The present study demonstrates a considerably higher rate and severity of gastrointestinal adverse effects secondary to zinc sulphate therapy in pediatric patients as previously reported. A total of 40% of our treated patients experienced gastrointestinal symptoms, of which the more severe cases were associated with endoscopically evident gastric ulcerations and erosions. Furthermore it was shown, that proton pump inhibitors were not effective in treating patients with severe zinc associated gastrointestinal side effects, requiring a switch to an alternative treatment regimen.